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Learn About Neuroblastoma
Written by NCI/PDQ®

Source: Cancer Resource Room

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What is Neuroblastoma?
Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue of the adrenal gland, neck, chest, or spinal cord.

Neuroblastoma often begins in the nerve tissue of the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen.

The adrenal glands produce important hormones that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Neuroblastoma may also begin in the chest, in nerve tissue near the spine in the neck, or in the spinal cord.

Neuroblastoma most often begins during early childhood, usually in children younger than 5 years. It sometimes forms before birth but is usually found later, when the tumor begins to grow and cause symptoms. In rare cases, neuroblastoma may be found before birth by fetal ultrasound.

By the time neuroblastoma is diagnosed, the cancer has usually metastasized (spread), most often to the lymph nodes, bones, bone marrow, liver, and skin.

What are the symptoms?
Possible signs of neuroblastoma include bone pain and a lump in the abdomen, neck, or chest.
The most common symptoms of neuroblastoma are caused by the tumor pressing on nearby tissues as it grows or by cancer spreading to the bone. These and other symptoms may be caused by neuroblastoma or by other conditions. A doctor should be consulted if any of the following problems occur:

  • Lump in the abdomen, neck, or chest.
  • Bulging eyes.
  • Dark circles around the eyes ("black eyes").
  • Bone pain.
  • Swollen stomach and breathing problems in infants.
  • Painless, bluish lumps under the skin in infants.
  • Weakness or paralysis (loss of ability to move a body part).

Less common signs of neuroblastoma include the following:

  • Fever
  • Shortness of breath
  • Feeling tired
  • Easy bruising or bleeding
  • Petechiae (flat, pinpoint spots under the skin caused by bleeding)
  • High blood pressure
  • Severe watery diarrhea
  • Jerky muscle movements
  • Uncontrolled eye movement
  • Swelling of the legs, ankles, feet, or scrotum

Tests that examine many different body tissues and fluids are used to detect (find) and diagnose neuroblastoma.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Twenty-four-hour urine test: A test in which a urine sample is checked to measure the amounts of certain substances. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease in the organ or tissue that produces it. A higher-than-normal amount of the substances homovanillic acid (HMA) and vanillyl mandelic acid (VMA) may be a sign of neuroblastoma.
  • Blood chemistry study: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease in the organ or tissue that produces it. A higher-than-normal amount of the hormones dopamine and norepinephrine may be a sign of neuroblastoma.
  • Cytogenetic analysis: A test in which cells in a sample of tumor tissue are viewed under a microscope to look for certain changes in the chromosomes.
  • Bone marrow aspiration and biopsy: The removal of a small piece of bone and bone marrow by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and the bone marrow samples under a microscope to look for abnormal cells.
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer.
  • X-ray: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
  • Ultrasound: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
  • Immunohistochemistry study: A procedure in which dyes or enzymes are added to a blood or bone marrow sample to test for certain antigens (proteins that stimulate the body's immune response).

Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:

  • Age of the child when diagnosed.
  • Stage of the cancer.
  • Where the tumor is in the body.
  • Tumor histology (the shape, function, and structure of the tumor cells).

Prognosis is also affected by tumor biology. In neuroblastoma, tumor biology is described according to the following:

  • The patterns of the tumor cells.
  • How different the tumor cells are from normal cells.
  • How fast the tumor cells are growing.
  • The number of chromosomes in the tumor cells.
  • How many copies of the N-myc gene there are.

The tumor biology is said to be favorable or unfavorable, depending on these factors. A favorable tumor biology means there is a better chance of recovery. To read more about Neuroblastoma >>>

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